Each month in “ You Make the Call ,” we pick a challenging clinical question submitted through the Consult a Colleague program and post the expert ’ s response , but we also want to know what you would do . Send in your response to next month ’ s clinical dilemma and see how your answer matches up to the expert ’ s in the next print issue .
This month , Ariela Noy , MD , discusses whether hypogammaglobulinemia alone is an indication for treatment of CLL .
CLINICAL DILEMMA :
I have a patient with newly diagnosed chronic lymphocytic leukemia ( CLL ) who has normal cytogenetics . The presenting features were fever and hypoxia that led to 2 weeks of hospitalization ; bronchoscopy revealed pneumocystis pneumonia . The patient is HIV negative and is now doing well without any typical criteria for CLL treatment . He has received intravenous immune globulin ( IVIG ) twice for IgG level of 200 mg / dL . Is hypogammaglobulinemia alone an indication for treatment for CLL , in the absence of other symptoms or cytopenias ?
Expert Opinion
Ariela Noy , MD Memorial Sloan-Kettering Cancer Center New York , NY
I see the patient ’ s infectious complications as having two parts : hypogammaglobulinemia and pneumocystis .
I find the case somewhat puzzling , as pneumocystis has more to do with T cell deficits than IgG status . I found a few case reports of treatment-naïve CLL patients who were diagnosed with pneumocystis , 1 , 2 but it is very rare and more often a complication of treatment with purine analogues , such as fludarabine . In the case reports , lung parenchymal infiltration may have contributed to mechanistic interference of immune response to pneumocystis . Regardless , I recommend determining the CD4 count and , if low , following them over time . Reconstitution will be necessary before discontinuation of any posttreatment pneumocystis prophylaxis .
Consult a Colleague Through ASH
Consult a Colleague is a service for ASH members that helps facilitate the exchange of information between hematologists and their peers . ASH members can seek consultation on clinical cases from qualified experts in 11 categories :
• Anemias
• Hematopoietic cell transplantation
• Hemoglobinopathies
• Hemostasis / thrombosis
• Lymphomas
• Lymphoproliferative disorders
• Leukemias
• Multiple myeloma & Waldenström macroglobulinemia
• Myeloproliferative neoplasms
• Myelodysplastic syndromes
• Thrombocytopenias
Hypogammaglobulinemia is quite common in CLL ; roughly 25 % of patients present with it at diagnosis , and 25 % develop it with time . 3 Typically , one does not treat with ongoing IVIG on that basis alone unless there are repeated viral or bacterial infections . It is not clear the hypogammaglobulinemia contributed to the pneumocystis . Given the patient ’ s unusual presentation , I would follow more closely than for asymptomatic CLL .
References
1 . Strich JR , Jerussi TD , Wiestner A , Holland , SM . Pneumocystis jirovecii pneumonia in a treatment-naive patient with chronic lymphocytic leukemia . Infect Dis Clin Pract ( Baltim Md ). 2016 ; 24 : e86 – e87 .
2 . Kalkanis A , Judson M , Napier M . Pneumocystis jirovecii pneumonia in a patient with untreated chronic lymphocytic leukaemia : a novel case and postulations concerning the mechanism . BMJ Case Rep . 2013 ; bcr2013202124 .
3 . Parikh SA , Leis JF , Chaffee KG , et al . Hypogammaglobulinemia in newly diagnosed chronic lymphocytic leukemia : natural history , clinical correlates , and outcomes . Cancer . 2015 ; 121:2883-2891 .
Assigned volunteers (“ colleagues ”) will respond to inquiries within two business days ( either by email or phone ).
Have a puzzling clinical dilemma ? Submit a question , and read more about Consult a Colleague volunteers at hematology . org / Clinicians / Consult . aspx or scan the QR code .
* If you have a request related to a hematologic disorder not listed here , please email your recommendation to ashconsult @ hematology . org so it can be considered for addition in the future .
Next Month ’ s Clinical Dilemma :
I have a 39-year-old female patient with polycythemia vera , who has required intermittent phlebotomy to keep hematocrit below 45 %. JAK2 V617F mutation is present at 34 % variant allele frequency . She has a persistently high white blood count and her highest neutrophil count has been 14,000 / mm 3 . She also has platelet count of 1.4 million /µ L . She has essential hypertension controlled with antihypertensive medication , and her myeloproliferative neoplasm symptom assessment score is 0 . What are the criteria to start cytoreductive therapy in a low-risk patient ?
How would you respond ? Email us at ashclinicalnews @ hematology . org .
18 ASH Clinical News November 2020